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Erratum. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis

Edward R. Smith

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Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease

Edward R. Smith

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Acute clival and spinal subdural hematoma with spontaneous resolution: clinical and radiographic correlation in support of a proposed pathophysiological mechanism

Case report

Edward S. Ahn and Edward R. Smith

✓Infratentorial and spinal subdural hematomas (SDHs) from traumatic injury in the pediatric population occur with such rarity that they can present the clinician with a challenge in diagnosis and management. When such hematomas are correctly identified, clinicians must decide whether to evacuate the lesion or manage it expectantly. The authors discuss the case of a 4-year-old child who presented with a clival and spinal SDH after a fall from a fourth-story window. The clinical and radiographic findings support a possible mechanism of evolution of these lesions. There is little evidence to guide management of clival and spinal SDHs. This case supports the evaluation for a spinal SDH when a clival hematoma is diagnosed. In the setting of a good neurological examination, expectant management can be an appropriate method of treatment. Additionally, this case lends insight into the pathophysiology of spinal SDHs. Unlike its intracranial counterpart, the spinal subdural space lacks bridging veins. The mechanism of formation of spinal SDHs after trauma has been heretofore relatively unclear. The images in this case support the hypothesis that redistribution of the clival SDH to dependent areas in the spinal subdural space is a significant mechanism in the evolution of these lesions.

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Editorial: Noninvasive biomarkers: are we there yet?

Jeffrey Ojemann

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Progression of disease in unilateral moyamoya syndrome

Edward R. Smith and R. Michael Scott


Progression of vasculopathy associated with moyamoya syndrome is extremely variable. The authors review their experience in patients with unilateral moyamoya syndrome to identify factors predictive of contralateral clinical and imaging-documented disease progression.


The authors reviewed the clinical and imaging records of all patients with moyamoya syndrome and unilateral disease who underwent cerebral revascularization surgery between January 1985 and June 2006 by using a standardized surgical procedure, pial synangiosis.


Of 235 surgically treated patients with moyamoya syndrome, 33 (14%) presented with unilateral disease (4 adults and 29 children). There were 16 female and 17 male patients, with an average age of 10.4 years (26.8 years for adults and 8.1 years for children; range 1.5–39 years). Twenty patients presented with left-sided disease and 13 with right-sided disease.

The average follow-up after surgery was 5.3 years (3.1 years for adults and 5.6 years for children; range 1–16 years). During this period, 10 (30%) of 33 patients progressed to bilateral disease. The mean time until disease progression was 2.2 years (range 0.5–8.5 years). Factors associated with progression in this series included contralateral abnormalities on initial angiography, previous history of congenital cardiac anomaly, cranial irradiation, Asian ancestry, and familial moyamoya syndrome. Young age at diagnosis was associated with a more rapid rate of progression (age < 7 years, 0.9 years to progression and age ≥ 7 years, 3.1 years to progression).


Of patients with unilateral moyamoya syndrome, 30% will have progression of arteriopathy during long-term follow-up. In this series, the average time of progression from unilateral to bilateral angiographic disease was 2.2 years. Several factors, including contralateral abnormalities on initial imaging, congenital cardiac anomaly, previous cranial irradiation, Asian ancestry, and familial moyamoya syndrome, were associated with an increased risk of progression. Patients with known unilateral angiographic disease should undergo continued monitoring by using MR imaging and MR angiography at regular intervals. Treatment with pial synangiosis is safe and confers durable protection against stroke in patients with both bilateral and unilateral moyamoya syndrome.

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Introduction: Moyamoya Disease

R. Michael Scott and Edward R. Smith

This issue of Neurosurgical Focus is devoted to the topic of moyamoya disease/syndrome. When the senior editor (R.M.S.) was a neurosurgical resident in the late 1960s and early 1970s, the condition was virtually unknown in the Western hemisphere, and patients with “cerebrovascular insufficiency” and the typical arterial findings on angiography were believed to have a type of arteritis. The refinement of catheter angiography techniques and the development of the imaging modalities of CT and MR imaging clarified the significance of making the correct diagnosis of moyamoya disease in affected patients, and with the development of direct and then indirect revascularization procedures during this same period, neurosurgeons became involved in the disease's treatment.

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Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines

A review

Edward R. Smith and R. Michael Scott

Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain (“moyamoya disease”) or can be unilateral or found in association with systemic disorders (“moyamoya syndrome”). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke—the primary presentations of affected patients. Although it is rare—affecting 1 in 1 million children in the US—moyamoya is implicated in 6% of all childhood strokes. Diagnosis is defined by characteristic findings on arteriograms, including stenosis of the branches of the ICA and a pathognomonic spray of small collateral vessels in this region, descriptively likened to a “puff of smoke” (“moyamoya” in Japanese). Treatment is predicated on restoration of cerebral blood flow by surgical revascularization. The rarity of this disorder has limited research and the development of evidence-based clinical management. While acknowledging these limitations, in this article the authors aim to summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based guidelines for treatment. The compilation of current data in these guidelines should serve as a resource to aid pediatric neurosurgeons in their role as advocates for providing appropriate care to affected children.

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Multifocal meningioangiomatosis in a 3-year-old patient

Case report

Osama Jamil, Shakti Ramkissoon, Rebecca Folkerth, and Edward Smith

Meningioangiomatosis consists of benign hamartomatous lesions of the brain and the leptomeninges, which typically present with seizure. Management is predicated on resection and control of seizures with medication. Lesions are typically solitary. Multifocal meningioangiomatosis is extremely rare, with only 2 cases reported in adults and none in children. The authors report the first case, to their knowledge, of multifocal meningioangiomatosis in a child. This unique case highlights therapeutic challenges associated with these lesions and demonstrates that multifocality is possible in the pediatric population. This finding has implications for diagnosis and follow-up for children afflicted with these tumors.

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Editorial. A terrible border wall: a study of pediatric moyamoya exposes socioeconomic barriers to care in the United States

Edward R. Smith

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Late morbidity and mortality following revascularization surgery for moyamoya disease in the pediatric population

Madeline B. Karsten, Edward R. Smith, and R. Michael Scott


There are limited reports on long-term morbidity in pediatric patients who have undergone surgical revascularization for moyamoya disease (MMD). Here, the authors report long-term morbidity and mortality in a population of pediatric patients who underwent pial synangiosis for MMD from 1988 through 2016.


A single-center retrospective review of the hospital and personal operative databases of the senior authors was carried out to identify all patients who were treated for MMD at Boston Children’s Hospital between 1988 and 2016, and who experienced any episode of late morbidity or mortality, which the authors defined as an event resulting in significant neurological deficit or death occurring more than 1 year after revascularization surgery. Hospital records were reviewed to determine pertinent demographic data, the initial mode of patient presentation, and associated comorbidities. Radiographic studies, when available, were reviewed for documentation of the diagnosis and for confirmation of the late complication, and the literature on this topic was reviewed.


In total, 460 patients with MMD underwent surgery between 1988 and 2016 using the pial synangiosis surgical technique; 15 (3.3%) of these patients (9 females and 6 males) experienced documented late death (n = 14) or severe morbidity (n = 1). The median age at revascularization surgery was 8.0 years (range 1–21 years). The causes of these late complications were grouped into three etiologies: intraventricular or intracerebral hemorrhage (n = 8), systemic complications related to associated comorbidities or preoperative disabilities (n = 5), and the development of malignant brain tumors (n = 2). Four patients whose MMD was associated with a history of cranial radiation therapy died. These events occurred from as early as 2 years to as late as 27 years postoperatively.


The risk of late morbidities and mortality following pial synangiosis for MMD in the pediatric patient appeared to be low. Nevertheless, the occurrence of catastrophic cerebrovascular events, particularly intracerebral and intraventricular hemorrhage in the otherwise neurologically stable revascularized patient, was concerning. Although there is value in long-term surveillance of patients who have undergone surgery for MMD, from both a neurological and a general medical standpoint, particularly in patients with the risk factor of prior cranial radiation therapy, it is not clear from the data how the late deaths in this population could have been prevented.